VITREORETINAL DEGENERATION AS A SIGN OF GENERALIZED CONNECTIVE-TISSUE DISEASES
VITREORETINAL DEGENERATION AS A SIGN OF GENERALIZED CONNECTIVE-TISSUE DISEASES
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DOI：
10.1016/0002-9394(79)90645-7
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发表时间：
1979-01-01
影响因子：
4.2
通讯作者：
MAUMENEE, IH
中科院分区：
文献类型：
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作者：
MAUMENEE, IH
显性玻璃体视网膜变性首先由瓦格纳描述为一种良性眼部疾病，现已广泛与伴有视网膜脱离的家族性视网膜变性过程相关。瓦格纳描述的家庭中没有人患有视网膜脱落。玻璃体视网膜变性，通常进展为脱髓鞘，也发生在几种不同的骨发育不良中。玻璃体视网膜变性的综合征的分类提供和结缔组织疾病，可能涉及II型胶原蛋白的迹象，其意义进行了讨论。在这个意义上，这种疾病类似于晶状体异位，是各种结缔组织疾病的标志。
Dominant vitreoretinal degeneration, first described by Wagner as a benign ocular condition, has become widely associated with a familial retinal degenerative process accompanied by retinal detachment. No one in the family described by Wagner suffered from a retinal detachment. Vitreoretinal degeneration, often progressing to detachments, occurs also in several distinct bone dysplasias. A classification of syndromes with vitreoretinal degeneration is provided and its significance as a sign of connective tissue diseases, probably involving type II collagen, is discussed. In this sense, the disorder resembles ectopia lentis as a sign of various connective tissue diseases.